Primary spinal extradural extraosseous primitive neuroectodermal tumor/Ewing's sarcoma: A critical analysis and review

Primary spinal extradural Ewing's sarcoma/primitive neuroectodermal tumor (PNET) is rare malignant tumor of childhood and early adulthood. The World Health Organization classifies PNET as an undifferentiated round cell tumor arising from primitive neuroepithelial cell. It can be central or peripheral PNET depending on site of presentation. Usually, the presenting symptoms are chronic back pain and myelopathy. Overall prognosis and survival are dismal in spite of total surgical resection and adjuvant therapy. Because of the rarity and malignant behavior, definite management of spinal PNET has never been described. After review of medical record at Acharaya Vinoba Bhave Rural Hospital, Sawangi, India, we identified four patients of spinal PNET and were included in our study. Age at diagnosis ranging from 15 to 26 years old with mean age of 20 years old. All four cases were epidural in location, two of which were of Askin type tumor with spinal cord compression. Rural population with low literacy and financial constraints were the key reasons of late presentations at our hospital. Counseling and proper education regarding the disease are a must for early case detection and early treatment of those living in rural areas and suffers from financial constraints. Due to rarity of the disease and its poor prognosis, a well-organized multicentric controlled trial is required to formulate a standard guidelines in the management of this disease.

Location
Deutsche Nationalbibliothek Frankfurt am Main
Extent
Online-Ressource
Language
Englisch

Bibliographic citation
Primary spinal extradural extraosseous primitive neuroectodermal tumor/Ewing's sarcoma: A critical analysis and review ; volume:16 ; number:02 ; year:2021 ; pages:276-280
Asian journal of neurosurgery ; 16, Heft 02 (2021), 276-280

Contributor
Patil, Akshay
Gupta, Prasheelkumar
Iratwar, Sandeep

DOI
10.4103/ajns.AJNS_340_20
URN
urn:nbn:de:101:1-2023012008054757231390
Rights
Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.
Last update
15.08.2025, 7:35 AM CEST

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Associated

  • Patil, Akshay
  • Gupta, Prasheelkumar
  • Iratwar, Sandeep

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